Right aortic arch with aberrant left subclavian artery and coarctation

Case Summary: A 12 year old girl presented to ‘well child clinic’ prior to sports participation with symptoms of occasional leg pain while running. A grade 2–3/4 murmur was present. Right upper extremity hypertension was present with the arm pressure measuring 159/68 mm Hg. Asymmetric extremity pulses were present with weak pulses in the left radial artery and bilateral dorsalis pedis pulses. A right aortic arch and coarctation was detected by echocardiography and MRI. MRI also detected a diverticulum of Kommerell and aberrant left subclavian artery (Fig. 1). Discussion: A right aortic arch is found in 0.1 % of the population. Ten patients (4 %) in a series of 240 patients with right aortic arch had coarctation, 6 of the ten also had an aberrant left subclavian artery [1]. Kommerell in 1936 described the aortic diverticulum associated with an aberrant right subclavian artery, from a left arch [2]. Kommerell’s diverticulum however can be associated with various anomalies of the aortic arch but is most frequent in cases of right arch with aberrant left subclavian artery [2]. Congenital heart defects associated with a right arch and coarctation include ventricular septal defect, double outlet right ventricle and pulmonary valve abnormalities [1].